Tumid Lupus Erythematosus - An Overview

Introduction

Cutaneous Lupus Erythematosus manifests in three distinct forms which are, acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE). Each form represents a different manifestation of the condition. Tumid lupus erythematosus (TLE), discoid lupus erythematosus (DLE), chilblain lupus erythematosus, and lupus panniculitis are the specific classifications of CCLE, representing distinct subtypes within the condition. Although the three forms differ in histological appearance, more than one form can co-exist in a person’s body. Although considered a subtype of cutaneous lupus erythematosus, TLE differs from the rest that it has no or less associations with systematic lupus erythematosus (SLE).

What Is Tumid Lupus Erythematosus?

Tumid lupus erythematosus is a rare inflammatory skin disorder characterized by erythematous plaques. It is a chronic photosensitive, erythematous, and edematous lesion affecting the head, neck, and trunk. The course and prognosis of the condition are generally more favorable than other subtypes of CLE. Tumid lupus erythematosus is usually seen as annular (round), indurated (having become firm due to an increase in fibrosis), erythematous (red), edematous (swollen or fluid-filled) papules, and plaques without epidermal involvement.

What Are the Causes of Tumid Lupus Erythematosus?

1. The exact cause of tumid lupus erythematosus has not been identified to date. Some factors trigger or aggravate the condition, like ultraviolet radiation.

2. The association of TLE with autoimmune diseases is still controversial. If the patient has any other autoimmune disease diagnosed, tests must be done.

3. Another hypothesis is immunity dysregulation with suppression of T cells.

4. Association of the condition with drugs like tumor necrosis factor antagonists, monoclonal antibodies, angiotensin-converting enzyme inhibitors, thiazide diuretics, and highly active antiretroviral therapy has been researched on.

5. Another factor that is thought to be a cause is smoking.

What Are the Symptoms of Tumid Lupus Erythematosus?

The symptoms of the condition may differ in the symptoms and severity from person to person. However, the most common symptoms can include;

• Cutaneous Photosensitivity: An increased skin sensitivity to light is termed cutaneous photosensitivity. The condition causes rashes on the skin when exposed to sunlight. This is called photodermatosis. It is tested by exposing a small area of the skin to light.

• Deep Pertivascular Inflammatory Infiltrate: An increased number of lymphocytes surrounding the blood vessels deep in the dermis are seen in this condition.

• Erythematous Plaque: A solid, raised lesion flat on the top and bigger than 1 cm in diameter is called a plaque. A red plaque is usually associated with inflammation or is caused by irritation.

• Presence of Antinuclear Antibodies: Autoantibodies will react against the nuclear components.

• Presence of Autoimmune Antibodies: An antibody in the blood that destroys the organism’s healthy cells are called an autoimmune antibody. In tumid lupus erythematosus, autoimmune antibodies in the serum are observed.

• Urticarial Plaque: A well-circumscribed pruritic (that causes itching) wound measuring about one to two centimeters in diameter is seen.

• Depigmentation or Hyperpigmentation: Some areas where the skin appears lighter in the shade and some areas where the skin is highly pigmented are seen.

• Skin Scaling: The outer layer of the skin is often flaky in tumid lupus erythematosus. It is called desquamation.

• Scarring: The lesions in TLE may not heal properly, leaving behind fibrous connective tissue on the unhealed or incompletely healed wounds. This is called scarring.

How Is Tumid Lupus Erythematosus Diagnosed?

The clinical manifestations of the skin, like the erythematous, infiltrated plaques without epidermal alterations seen in areas exposed to the sun, can point out the condition or helps to suspect TLE. The diagnosis can be established through many clinical and laboratory tests.

1. Physical Examination: A thorough examination will show erythematous lesions without epidermal changes. In addition, lymphadenopathy (enlargement of the lymph nodes) or arthritis (inflammation and joint pain) can be seen.

2. Punch Biopsy: A small circular tissue section is extracted from the affected area using a specialized procedure during a punch biopsy. It is usually about the size of a pencil eraser and is taken using a sharp hollow circular instrument. A biopsy is taken from the active site (erythematous lesion). A punch biopsy of 4mm is recommended on the trunk and 3mm on cosmetic areas like the face. It should be done to include the full layer of the dermis. Histopathological findings can include abundant interstitial mucin and a dermal perivascular and peri-adnexal lymphocytic infiltrate.

3. Direct Immunofluorescence (DIF): A lesional biopsy can be sent for direct immunofluorescence in conditions where the histology correlates with lupus but is not definitive of TLE. In tumid lupus s, DIF is often negative or nonspecific. Hence, it can distinguish TLE from the other forms of cutaneous lupus erythematosus.

4. Photo Testing: Testing photosensitivity using this test helps diagnose TLE. Photosensitive lesions will produce rashes on exposure to sunlight.

How Is Tumid Lupus Erythematosus Treated?

Photoprotection (or protection from sunlight), along with topical or intralesional corticosteroids, is usually the first line of treatment for the condition. Using a water-resistant sunscreen with a sun protective factor of 30 or above, sun protective clothing, avoiding peak sun exposure, and stopping or quitting smoking is recommended. Topical application of corticosteroids on the lesions is advised for two to four weeks.

An improvement in the condition is seen within two weeks after starting the treatment. The lesions on the face are treated using low-potent corticosteroids, while high-potent corticosteroids are used for the ones on the trunk and other parts of the body. The adverse effects of corticosteroids can include cutaneous atrophy (marked hypoplasia and loss of skin elasticity), striae, hypopigmentation, and telangiectasias (small dilated blood vessels on the skin).

What Are the Differential Diagnoses of TLE?

The differential diagnoses of TLE can include:

• Lymphocytic infiltrate of Jessner.

• Polymorphic light eruption.

• Pseudolymphoma of the skin.

• Reticular erythematous mucinosis.

• Granuloma fasciale.

Conclusion

The condition has a benign course. The solitary lesions are usually self-limiting and, in most cases, may not require topical or systemic therapy. Recurrence of the lesions is noted in disease-free periods. TLE usually shows a good prognosis than the other types of cutaneous lupus erythematosus. In many patients without symptoms, protection from sunlight alone proved to cure or resolve the lesions.